Potential role of thrombelastography in the monitoring of acquired factor VIII inhibitor hemophilia A: report on a 78-year-old woman with life-threatening bleedings.
A 78-year-old woman was admitted to our hospital because of syncope associated with hematomas in both legs. Acquired hemophilia A (AHA) with a low antifactor VIII antibodies activity was diagnosed. Whole blood (WB) thrombelastographic profile depicted a hypocoagulable state. During hospitalization, the patient experienced life-threatening bleedings in the neck and in the right thigh. FVIII concentrates and rFVIIa was safe and effective in controlling acute hemorrhagic symptoms. Immunosuppressive therapy was used successfully to eradicate the inhibitor. At discharge, FVIII inhibitor was absent and thrombelastogram showed a normal profile. Our report confirms that AHA is a heterogeneous condition in terms of risk of bleeding. Even though the criteria for the diagnosis of AHA is quite well defined, a laboratory test useful to predict the bleeding risk and monitor the response to treatment is lacking. ROTEM profile appears to be correlated with the response to treatment and with the eradication of the inhibitor.
Spiezia
Luca
L
Department of Medical and Surgical Sciences, 2nd Chair of Internal Medicine, University of Padua Medical School, Via Ospedale 105, Padua, Italy.
Meneghetti
Leonardo
L
Dalla Valle
Fabio
F
Tognin
Giulio
G
Radu
Claudia
C
Saggiorato
Graziella
G
Fadin
Mariangela
M
Zanon
Ezio
E
Simioni
Paolo
P
D000208:Acute Disease; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D016903:Drug Monitoring / Q000379:methods; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000276:immunology*; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000276:immunology*; D006470:Hemorrhage / Q000097:blood / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011994:Recombinant Proteins / Q000627:therapeutic use; D012720:Severity of Illness Index; D013916:Thrombelastography
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa